• Fang Sha, Xiujuan Du, Yane Gao, Beibei Wang, Xuan Sun, Kai Tang, and Hongsheng Bi.
• Shandong University of Traditional Chinese Medicine, Jinan, China.
• Medicine (Baltimore). 2025 Jan 17; 104 (3): e41239e41239.

RationaleCongenital ectropion uveae (CEU) is a rare, nonprogressive anomaly characterized by the proliferation of the iris pigment epithelium on the anterior surface of the iris, often associated with glaucoma. Due to its rarity and complexity, standardized glaucoma surgical management is limited. To our knowledge, the application of glaucoma drainage devices in CEU is rarely documented. Here, we report a case of Ahmed glaucoma valve (AGV) implantation for unilateral CEU associated with glaucoma.Patient ConcernsA 26-year-old female initially presented with blunt ocular trauma with an intraocular pressure (IOP) of 37 mm Hg in the right eye. After starting glaucoma medication, IOP promptly decreased to 21 mm Hg. However, the patient was subsequently lost to follow-up.DiagnosesA definitive diagnosis was not made due to the limited understanding of CEU at the first visit. Nearly 3 years later, the patient was referred to our hospital again with decreased vision for 6 months and mild distending pain in the right eye. The best-corrected visual acuity was 20/25, IOP was 51 mm Hg, and the cup-to-disc ratio was 0.8. Slit-lamp examination of the right eye revealed 360° ectropion uveae, extending around the pupil to the mid-periphery of the iris, which was an unaltered condition since the first visit. Thus, the patient was diagnosed with CEU and unilateral glaucoma.InterventionsThe AGV implantation surgery with mitomycin C was performed in the right eye.OutcomesThe best-corrected visual acuity of the right eye improved to 20/20. IOP stabilized without medications during the entire period of follow-up for 3 years.LessonsAlthough CEU is rare, ophthalmologists should remain vigilant to avoid missed diagnoses due to its high association with glaucoma. AGV implantation with mitomycin C may be considered an effective surgical management for adult patients with late-onset glaucoma secondary to CEU.Copyright © 2025 the Author(s). Published by Wolters Kluwer Health, Inc.

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